Diffuse Large B Cell Lymphoma of Lacrimal Drainage System : A Rare Case Report

Abstract

Tumours of lacrimal drainage system are a rare entity and among these rare tumours lymphomas are a rarity. We report a case of 65 year old male who presented with mass inferior to left lacrimal sac region which was diagnosed as Diffuse Large B Cell Lymphoma on histopathology and immunochemistry. The patient underwent excision biopsy followed by chemotherapy and is disease free till writing of this report.

Introduction

The tumours of lacrimal drainage system (LDS) are rare. About 90% of these tumours are epithelial in origin and incidence of lymphoma in this area is very rare [1]. Majority of the cases lymphomas in this region are due to systemic metastasis of the lymphoma [2]. Squamous cell carcinoma is the most prevalent type of tumor, followed by transitional cell and occasionally adenocarcinoma, adenocystic carcinoma and mucoepidermoid carcinoma [3–7]. In this study we report a case of diffuse large cell lymphoma of lacrimal drainage system which presented as a swelling in the left lacrimal sac region.

Case Report

A 65-year-old male patient presented with complains of swelling over his left cheek over the past two months and increased lacrimation from left eye since one month. On examination a 2 × 2 cm swelling was present just inferior to left lacrimal sac region. On palpation the mass was firm and not mobile. Diagnostic nasal endoscopy was done which was unremarkable on both sides.

The patient underwent contrast-enhanced computed tomography of nose, paranasal sinus and orbits (Fig. 1). The CT demonstrated the presence of an enhancing soft tissue mass of size 28 × 16 mm in the region of the left nasolacrimal duct that was extending into the left maxillary sinus, anteriorly into the premaxillary fat region with thinned out frontal process of maxilla on left side. The anterior and medial wall of left maxillary sinus adjacent to nasolacrimal duct region was also found to be dehiscent, along with dehiscence of inferomedial margin floor of left orbit.

Fig. 1.

CT image showing mass in left lacrimal drainage system with dehiscence of medial side floor of orbit

The patient underwent fine needle aspiration cytology, which revealed an atypical lymphoproliferative disease. He was planned for excision biopsy of the mass. A lateral rhinotomy incision was given and tumor was removed (Fig. 2).

Fig. 2.

Intraoperative image showing mass in left nasolacrimal system

Histopathological findings were consistent with High Grade B Cell Lymphoma. Immunohistochemistry studies found positive expressions for CD20 (4+), PAX-5 (4+), BCL-2 (4+), BCL-6 (2+), and Ki-67 (60–70%) and based on this diagnosis of Diffuse Large B Cell Lymphoma was made (Fig. 3).

Fig. 3.

Histopathology consistent with High Grade B Cell Lymphoma

Patient underwent whole body positron emission tomography scan which revealed no evidence of distant metastases. The tumor was classified as stage 2.

He was treated with three cycles of chemotherapy based on Rituximab, Cyclophosphamide, Doxorubicin, and Vincristine. The patient was on follow-up schedule for six months and there are no signs of any recurrence till the writing of this report (Fig. 4).

Fig. 4.

Patient at six month follow up with no recurrence

Discussion

Tumors of lacrimal drainage system are rare. Most of these patients present with epiphora or mass inferior to the medial canthus. Some of these patients can also present with symptoms of acute dacryocystitis. Patients with advanced disease can report with orbital extension and restriction of ocular movements [8]. Contrast enhanced CT of paranasal sinus with orbits is very informative in these cases. Large tumors with orbital or intracranial extension may require Magnetic Resonance Imaging [8].

There are few neoplastic conditions that can clog the nasolacrimal duct, including papillomas, lymphomas, squamous cell carcinomas, and melanomas [9]. The majority of lymphomas that do manifest as epiphora typically start inside the lacrimal sac [10].The patient in this study presented atypically with lymphoma in the nasolacrimal duct system.

There are many prognostic indicators for lymphoma patients. The factors with poor prognosis are advanced age, stage 4 disease, elevated lactate dehydrogenase, high grade histological subtype, bone marrow involvement, presence of B symptoms (fever, weight loss and night sweats) [11].

Presence of swelling in the pre-maxillary region is usually suggestive of a maxillary sinus pathology but in present case, it turned out to be a nasolacrimal duct pathology for which patient must be investigated extensively using the radiological means. Post operatively the resected specimen must undergo histopathological examination and immunohistochemistry to know the exact characterization of the tumor so that an adequate chemotherapy can be given to these patients. Currently, radiotherapy or chemoradiotherapy is administered after a surgical resection of these tumors. The treatment of tumors of the lacrimal drainage system involves a broad surgical resection due to its closeness to the orbit and skull base. Based on the tumor’s penetration of the orbital tissue, the choice to perform orbital exenteration is sometimes made on an individual basis [12].

Conclusion

Lymphomas of the lacrimal drainage system are rare. They have good prognosis if diagnosed in early stage. Elderly patients with mass near medial canthus of eye with epiphora should raise the suspicion of lacrimal drainage system tumors. Contrast enhanced CT plays an important role in diagnosis. Delay in diagnosis can lead to intra orbital spread of the tumor.